Malignant gangliocytic paraganglioma of the duodenum with distant metastases and a lethal course

Gangliocytic paraganglioma (GP) is rare and has been regarded as benign in general with a good prognosis. We present a patient with duodenal GP showing a malignant and lethal clinical course. A 47-year-old male patient was found to have a duodenal tumor and enlarged regional lymph nodes. The patient initially underwent a pancreaticoduodenectomy to resect the tumor and involved lymph nodes completely. Histological and immunohistochemical analyses showed findings typical of GP. However, the distant metastatic lesions in the liver and pelvic cavity were rapidly observed after surgery. The patient underwent chemotherapy and radiotherapy, as well as a second surgery to partly remove the metastatic mass in the pelvic cavity. The histological examination revealed no significant difference in histological features between the primary duodenal tumor and the metastatic pelvic mass. However, the patient finally died of the tumor due to the recurrence of the residual pelvic lesion and increased liver mass. To our knowledge, this is the first report of lethal GP with multifocal metastases. Our case confirms that GP should be regarded as a malignant potential tumor with behavior code of "1", rather than a benign tumor of "0".

Medienart:

E-Artikel

Erscheinungsjahr:

2014

Erschienen:

2014

Enthalten in:

Zur Gesamtaufnahme - volume:20

Enthalten in:

World journal of gastroenterology - 20(2014), 41 vom: 07. Nov., Seite 15454-61

Sprache:

Englisch

Beteiligte Personen:

Li, Bin [VerfasserIn]
Li, Yang [VerfasserIn]
Tian, Xiao-Ying [VerfasserIn]
Luo, Bo-Ning [VerfasserIn]
Li, Zhi [VerfasserIn]

Links:

Volltext

Themen:

Biomarkers, Tumor
Case Reports
Duodenal neoplasms
Gangliocytic neoplasms
Journal Article
Lymphatic metastasis
Paraganglioma
Review
Treatment outcome

Anmerkungen:

Date Completed 10.08.2015

Date Revised 08.04.2022

published: Print

Citation Status MEDLINE

doi:

10.3748/wjg.v20.i41.15454

funding:

Förderinstitution / Projekttitel:

PPN (Katalog-ID):

NLM243440669